Ugras Ali Akin, Sungur Ibrahim, Akyildiz Mustafa Fehmi, Ercin Ersin
Department of Orthopedics, Haseki Training and Research Hospital, Istanbul, Turkey.
Orthopedics. 2010 Feb;33(2):124-6. doi: 10.3928/01477447-20100104-30.
Femoral bifurcation and tibial agenesis are rare anomalies and have been described in both the Gollop-Wolfgang complex and tibial agenesis-ectrodactyly syndrome. This article presents a case of Gollop-Wolfgang complex without hand ectrodactyly. Tibial agenesis-ectrodactyly syndrome and Gollop-Wolfgang complex are variants of tibial field defect, which includes distal femoral duplication, tibial aplasia, oligo-ectrodactylous toe defects, and preaxial polydactyly, occasionally associated with hand ectrodactyly.This article describes the case of a patient with bilateral tibial hemimelia and left femoral bifurcation. The proximal tibial anlage had not been identified in the patient's left leg. After failed fibular transfer procedure, the knee was disarticulated. The other leg was treated with tibiofibular synostosis and centralization of fibula to os calcis. At 7-year follow-up, the patient ambulates with an above-knee prosthesis and uses an orthopedic boot for ankle stability.In patients with a congenital absence of the tibia, accurate diagnosis is of the utmost importance in planning future treatment. In the absence of proximal tibial anlage, especially in patients with femoral bifurcation, the knee should be disarticulated. Tibiofibular synostosis is a good choice in the presence of a proximal tibial anlage and good quadriceps function.
股动脉分叉和胫骨发育不全是罕见的异常情况，在戈洛普 - 沃尔夫冈综合征和胫骨发育不全 - 缺指（趾）畸形综合征中均有描述。本文介绍了一例无手部缺指（趾）畸形的戈洛普 - 沃尔夫冈综合征病例。胫骨发育不全 - 缺指（趾）畸形综合征和戈洛普 - 沃尔夫冈综合征是胫骨区域缺陷的变体，其包括股骨远端重复、胫骨发育不全、少指（趾）畸形足趾缺陷和轴前多指（趾）畸形，偶尔伴有手部缺指（趾）畸形。本文描述了一名患有双侧胫骨半肢畸形和左侧股动脉分叉患者的病例。在患者左腿中未发现近端胫骨原基。腓骨转移手术失败后，膝关节被离断。另一条腿接受了胫腓骨融合术以及腓骨向跟骨的中心化手术。在7年的随访中，患者使用膝上假肢行走，并使用矫形靴来保持踝关节稳定。对于先天性胫骨缺失的患者，准确诊断对于规划未来治疗至关重要。在没有近端胫骨原基的情况下，尤其是在患有股动脉分叉的患者中，应离断膝关节。在存在近端胫骨原基且股四头肌功能良好的情况下，胫腓骨融合术是一个不错的选择。
